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Atrial myxoma - Skin
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Atrial myxoma - Skin

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Contributors: Marie Crisel B. Erfe MD, Noah Craft MD, PhD, Art Papier MD, William Van Stoecker MD
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Synopsis

Cardiac myxoma is the most common benign primary tumor in the heart. It represents 75% of all primary neoplastic masses identified pathologically in the heart. It can occur in all age groups and both sexes, but it is most common in adults between the third and sixth decade of life and in women. Clinical symptoms are variable and are dependent on tumor size, location, and mobility. The condition manifests with one or more signs and symptoms from the classic triad of intracardiac obstruction, extracardiac embolism, and systemic / constitutional complaints.

Cutaneous manifestations of atrial myxomas are non-specific and can be classified into embolic cutaneous signs, non-embolic signs associated with autoimmune symptoms, and cutaneous signs of a complex syndrome. Solitary cutaneous myxomas have also been described, but are thought to be distinct entities from cardiac myxomas.

75% to 80% of cardiac myxomas are localized in the left atrium, 15% to 20% are localized in the right atrium, and 10% are either biatrial or in the ventricles. Myxomas most commonly develop from the interatrial septum but can also originate from the posterior and anterior atrial walls or the atrial appendage.

Dyspnea, recurrent pulmonary edema, and right heart failure may suggest obstructed filling of the right or left ventricles. Auscultatory findings include arrhythmias such as atrial fibrillation and systolic and diastolic murmurs depending on tumor location and mobility. Right atrial involvement may present with pericardial friction rub, and left atrial tumors present with loud and widely split S1. Large myxomas obstructing the mitral or tricuspid valve may lead to syncope and sudden death.

Constitutional manifestations include fever, fatigue, erythematous rash, arthralgia, myalgia, and weight loss. Raynaud's phenomenon, polymyositis, hepatic dysfunction, and hyperglobulinemia may also occur. These symptoms are attributed to an autoimmune response or secondary infection from the tumor.

Most cases arise sporadically. Familial cases have been postulated as autosomal dominant inheritance with a variable phenotype and are associated with the occurrence of multiple and atypical tumors. Complex familial tumors are part of a rare condition called the Carney complex. Carney complex may manifest with adrenocortical involvement, pituitary-related symptoms, abnormal pigmentation, or multiple neoplasias in the skin, thyroid, testicles, breast, and neural systems. Heterozygous mutation in the PRKAR1A gene, a protein kinase A regulatory subunit-1-alpha gene, on chromosome 17q22-24 has been linked to cases of intracardiac myxoma and to Carney complex type 1. Carney complex type 2 involves a locus at chromosome 2p16, although the gene responsible remains unknown.

Cardiac myxomas can grow rather quickly. Recurrence is thought to be due to inadequate resection, multicentric growth, inheritance, or metastasis and usually happens during the first 4 years post-excision. Sporadic cases almost never recur, while incidence of recurrence for familial cases, Carney complex, and multiple myxomas are 10%, 22%, and 33%, respectively.

Codes

ICD10CM:
D15.1 – Benign neoplasm of heart

SNOMEDCT:
233854003 – Atrial myxoma

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Cutaneous manifestations:
Intracardiac thrombi – Most common differential diagnosis; often occurs in patients with atrial fibrillation. Diagnose by cardiac magnetic resonance imaging (MRI) or coronary angiography.

Benign primary cardiac tumors:
  • Papillary fibroelastomas – Second most common benign cardiac tumor; occurs predominately in elderly patients (>80 years old). Usually less than 1 cm in diameter, sessile pedunculated tumors attached to the mitral valve or semilunar valves; occurs exclusively on endocardium.
  • Rhabdomyomas – Most common cardiac tumor in children; often multiple masses located in the ventricular myocardium; 50% of cases are associated with tuberous sclerosis.
  • Fibromas – Congenital tumors seen in children. Usually solitary, fibrous, and calcified; located in the myocardium.
  • Hemangiomas – Highly vascularized.
  • Lipomas – Encapsulated tumors usually originating from the epicardium.
  • Paragangliomas
  • Atrioventricular node tumor – Located at the base of the atrial septum.
Malignant primary cardiac tumors – Tend to be large, lobular with ill-defined borders, and broad-based and show evidence of tissue invasion, necrosis, and calcifications.
  • Angiosarcoma – Highly invasive; usually with evidence of lung, brain, and/or liver metastasis upon diagnosis.
  • Rhabdomyosarcoma – Usually diagnosed in children; highly aggressive and may occur in any heart chamber.
  • Osteosarcoma – Bulky, sessile tumors usually located in the left atrium.
  • Leiomyosarcoma – Highly invasive; sessile masses with mucoid surface.
  • Lymphoma – Associated with lymphoproliferative conditions such as AIDS and in immunocompromised patients.
Metastatic tumors – From direct extension from tumors in the mediastinum; hematogenous or lymphatic spread; more commonly affects the pericardium or myocardium.

Valvular vegetations

Right atrial masses – Prominent eustachian valve; aneurysms of the interatrial septum.

Carney complex

McCune-Albright syndrome

Peutz-Jeghers syndrome

Cushing's syndrome with primary pigmented nodular adrenocortical disease (PPNAD)

Cardiac myxomas should always be considered as a differential in cases of pulmonary embolism and diagnosed definitively by histological evaluation.

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Therapy

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References

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Last Updated: 07/24/2014
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Atrial myxoma - Skin
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Atrial myxoma (Constitutional Symptoms) : Fatigue, Fever, Heart murmur, Splinter hemorrhage, Weight loss, Arthralgia, Peripheral leg edema, Dyspnea
Clinical image of Atrial myxoma
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